Vascular Dan Flashcards
How is ABPI performed?
How is it interpreted?
Systolic BP in ankle (highest of either DP or PT) ÷ systolic BP in arm (brachial arteries) while patient is in resting supine position for right and left
Normal 0.91 to 1.30
PVD at rest ≤ 0.9
Mild to mod PVD 0.41-0.90
Severe PVD ≤0.4
Falsely elevated ABI can occur in some limbs if vessels are very calcified and fail to compress - diabetes, arterioclerosis
Need ABPI >0.8 to undergo compression therapy
What is Thromboangitis Obliterans (Buergers disease)?
How is it treated?
Type of small vessel vasculitis affecting the upper limb and sometimes the lower limb
As names suggests there is thrombosis and luminal occlusion followed by fibrosis of vessels
Esp young men who are heavy smokers esp middle and Far East, and Indian subcontinent
Causes claudication, rest pain, red/cyanotic acral colour changes and can develop chronic painful paronychia or ischaemia esp of digits
Claudication in arch of foot is characteristic a sis intolerance to COLD
Mx
Essential to stop smoking
Bosentan can help
Vasc surgeon and physician
What is erythromelalgia?
what are key features and major types?
Episodic attacks of erythema and burning pain of extremety during which skin feels hot to touch
Attacks are precipitated by exercise or heat and are relieved by rapid cooling. Lasts from a few minutes to several hours and can become almost continuous
Cause unknown, some familial AD cases
Can be primary (idiopathic) or secondary
secodnary Dx due to thrombocythaemia is usually unilateral and may be relieved by aspirin
Other secondary types related to PVD or CTD eg SLE or rarely tiggered by drugs; Ca channel blockers, CsA
What are diagnostic criteria for erythromelalgia?
BARE Heat
- Burning pain in extremities
- pain Aggravated by warming
- pain Relieved by cooling
- Erythema of affected skin
- increased temperature of affected skin
What is the prognosis and management of erythromelalgia?
30% each: worsening, no change, improvement;
+ 10% complete resolution
No effective treatment and it gets worse with time.
Avoid exacerbating factors
Thrombocythaemia type may be relieved by aspirin + may give some relief in other types
Elevation to decrease oedema
Clopidogrel
Prostaglanding E infusion or oral misoprostol (prostaglandin E analogue)
Try propranolol, amitriptyline, gabapentin, SSRI’s
T/F
telangiectasia are new vessels
F
Dilations of pre-existing vessels without any new vessel growth
T/F
spider angiomas/naevi, angiokeratomas and angiomas and including cherry angiomas are all types of telangiectasia
T
What are the causes of Spider Telangiectasias?
How are they treated?
Idiopathic - common; 15% normal population esp kids
pregnancy (disappear after delivery) and OCP use
Liver disease
Rx
reassure - esp if pregnant (recur in later pregs)
treat cause if possible
Fine tip hyfrecator
Laser - PDL, KTP
2mm punch excise
What are causes of primary telangiectasia?
AASONG Ataxia telangiectasia Angiomas, Angiokeratomas, Angioma serpiginosum Spider naevi Osler weber rondu (HHT) Naevi; • Naevus anaemicus • CMTC Generalised essential telys and variants e.g. • Unilateral naevoid telangiectasia • Hereditary benign telangiectasia
What are causes of secondary telangiectasia?
EXTRACT and varicose veins Environmental; - solar, XRT XP Trauma (scar) Raynauds, Rosacea Atrophy (TCS, poikiloderma), AIDS CREST/Scleroderma/morphoea/LE*/Dermatomysoitis, Ca channel bockers TMEP * inc lupus erythematosis telangiectoides and rosacea-like lupus (both DLE variants)
What is solitary circumscribed angiokeratoma?
Small warty black papule on legs mainly (but can occur anywhere)
may be several
Result from trauma or chronic irritation of venule wall
Can be confused with melanoma
What is Angiokeratoma circumscriptum?
Plaque of multiple discrete coalescing papules, or hyperkeratotic papules and nodules that become confluent
Arise on trunk or limbs usually unilaterally in childhood
Resembles pseudokaposis sarcoma
Benign; cosmetic treatments
What is Angiokeratoma of Mibelli?
AD with variable penetrance
develop agminated region of flat or papular red angiokeratomas on dorsal/lateral aspects of fingers/toes (also dorsa of hands and feet and rarely elbows and knees)
onset age 10-15
May be chilblains and acrocyanosis
What are the associations of Angiokeratoma of Fordyce in men and women?
M
thrombophlebitis, varicoeles, inguinal hernias
F
vulvar varicosities, haemorrhoids, OCP’s or increased venous pressure in pregnancy
What conditions are associated with Angiokeratoma corporis diffusum?
Fabry's most characteristically; XLR α-galactosidase A deficiency Fucosidosis Sialidosis Galactosialidosis Multiple others
What causes eruptive cherry angiomas?
assoc w/ lymphoproliferative disease eg multicentric Castleman’s disease
T/F
angiomas are more common in middle and old age but dissappear in extreme old age
T
What are venous lakes?
where do they occur?
Greatly dilated thin walled venules without proliferation of vascular tissue
Due to degeneration of supporting connective tissue
face, lips, and ears of elderly patients
Rx
Cryo - conatct freeze to empty with pressure
Hyfrecate
Laser - NdYAG preferred; can use diode, Alex, Ruby
Excision
What is Angioma serpiginosum?
Rare benign naevoid disorder
females (90%) in childhood
grouped areas or sheets of tiny angiomas
can be mistaked for petechiae but blanch (although not completely) and magnification shows vessels
lower limbs, buttocks; starts unilateral and often becomes bilateral
Can spontaneously resolve or persist
treat w/ vasc laser
What is Hereditary Haemorrhagic Telangiectasia (Osler-Rendu-Weber syndrome)?
AD disease causing telangiectasia, epsitaxis and visceral vascular malformations
2 types w/ different genes affected and slightly different risks;
HHT1 or endoglin gene (ENG) = higher risk of pulmonary and cerebral AVMs
HHT2 or activin receptor-like kinase 1 gene (ACVRL1) = higher risk of liver AVMs
What are diagnostic criteria for Hereditary Haemorrhagic Telangiectasia (Osler-Rendu-Weber syndrome)?
For definite diagnosis need all 3 of;
Epistaxis - onset around age 12
Telangiectasias - onset teens/after puberty
Visceral AV malformations
If 2 present is suspected diagnosis - highly likely if family history
What are features of the telys in HHT?
Onset at/after puberty anywhere but usually upper half of body esp face Mucosa usually involved Often lips, oral cavity, fingers, nose skin lesions rarly bleed
What is the management of pt with HHT/suspected HHT?
Hx
nose bleeds, tiredness/SOB(anameia), haemoptysis, cyanosis, malena, stroke
FHx - ?known HHT
consider other causes of telys
Ix and make referrals;
FBC – low Hb and haematocrit - microcytic/normocytic anemia in GIT AVMs
Fe studies – iron deficiency
FOB – positive in GIT AVMs
TTE(bubble study) – detect pulmonary AVMs which lead to right-to-left shunting
HRCT lungs, CXR
Brain MRI – cerebral AVMs
Upper endoscopy/capsule endoscopy
Colonoscopy
Doppler USS or CT liver – hepatic AVMs
Genetic testing
Mx
Education very important
PDL/IPL for telangiectasia
Iron for anaemia +/- RBC transfusions
Antibiotic prophylaxis for procedures with risk of bacteremia
Precautions to avoid air bubbles during IV cannulation
Avoid scuba diving
Be aware of epistaxis/internal bleeding- can be a big emergency!
Refer for lifelong screening program for pt and family to detect AVMs of brain or lung
Ca need liver Tx if sever liver disease
Disease does not usually shorten life; mortality
What are the main features of Ataxia telangiectasia?
Rare, AR mutation in ATM gene; AAATM
AR, Progressive cerebellar ataxia, low IgA
Telangiectases - face, conjunctivae>trunk>hand/ feeet
Marked susceptibility to cancer, particularly lymphoma and leukaemia
- Combined immunodeficiency w/ low IgA
Apart from telys what skin findings are seen in Ataxia telangiectasia?
90% get Progeric changes of skin and hair - Grey hair, fat atrophy on face
Granulomatous plaques on skin that ulcerate
Nevoid hyper- and/or hypopigmentation (large segmental CALMs)
Poikiloderma
Eczematous dermatitis
Seborrheic dermatitis
KP
Vitiligo
Acanthosis nigricans
T/F
Pts with Ataxia telangiectasia should avoid ionizing radiation including medical imaging
T
increased sensitivity to ionizing radiation and cancer risk
keep scans to minimum and chose MRI if possible
What is cause of naevus anaemicus?
Congenital pale patch 5-10cm esp on trunk
Within the patch the blood vessels are permanently vasosonstricted due to sensitivity to endogenous catecholamines
May have islands of sparing within
Histo normal
